Inherited haemoglobin disorders, including thalassaemia and sickle-cell disease, are the
most common monogenic diseases worldwide. Several clinical forms of α-thalassaemia and …

Abstract β-Thalassemia is caused by reduced (β+) or absent (β 0) synthesis of the β-globin
chains of hemoglobin. Three clinical and hematological conditions of increasing severity are …

Non-transfusion-dependent thalassemias include a variety of phenotypes that, unlike
patients with beta (β)-thalassemia major, do not require regular transfusion therapy for …

Thalassemia syndromes are among the most serious and common genetic conditions. They
are indigenous in a wide but specific geographical area. However, through migration they …

Iron homeostatic mechanisms are key to the pathophysiology of transfusional iron overload
(TIO). In humans, these mechanisms are best adapted to increasing iron acquisition in …

Background Patients with β thalassemia intermedia can have substantial iron overload,
irrespectively of their transfusion status, secondary to increased intestinal iron absorption …

Thalassemia syndromes are characterized by the inability to produce normal hemoglobin.
Ineffective erythropoiesis and red cell transfusions are sources of excess iron that the human …

Our understanding of the molecular and pathophysiological mechanisms underlying the
disease process in patients with β-thalassemia intermedia has substantially increased over …

Iron overload due to increased intestinal iron absorption represents an important clinical
problem in patients with non-transfusion-dependent thalassemia (NTDT), particularly as they …

Patients with non-transfusion-dependent thalassemia (NTDT) experience many clinical
complications despite their independence from frequent transfusions. Morbidities in NTDT …